Medication Summary

Therapy for idiopathic restrictive cardiomyopathy (RCM) is limited to symptomatic treatment and is often ineffective in improving outcome.

Class Summary

Diuretic agents promote excretion of water and electrolytes by the kidneys. Treatment with diuretics may improve symptoms of venous congestion. However, these agents should be used with caution, because some patients require high venous filling pressures to maintain adequate cardiac output.

Furosemide (Lasix)

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Furosemide is a loop diuretic that blocks the sodium-potassium-chloride transporter and works primarily on thick ascending limb of the loop of Henle. It also inhibits sodium and chloride absorption from the proximal and distal tubules.

Chlorothiazide (Diuril)

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Chlorothiazide is a thiazide diuretic that blocks the electroneutral sodium-chloride transporter.

Metolazone (Zaroxolyn)

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Metolazone is a quinazoline diuretic with properties similar to those of thiazide diuretics. It inhibits sodium resorption at the cortical diluting site and the proximal convoluted tubule.

Spironolactone (Aldactone)

Spironolactone is an aldosterone antagonist that spares potassium. It competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Class Summary

Some authors advocate the use of anticoagulation, antiplatelet agents, or both in children with RCM because of due to the high reported incidence of thromboembolic events in several small case series. If chosen, anticoagulation agents should be carefully administered with close supervision of coagulation parameters.

Heparin

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Heparin augments the activity of antithrombin III and prevents conversion of fibrinogen to fibrin. It does not actively lyse but is able to inhibit further thrombogenesis. It prevents reaccumulation of clot after spontaneous fibrinolysis.

Warfarin (Coumadin)

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Warfarin interferes with hepatic synthesis of vitamin K–dependent coagulation factors. It is used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.

Enoxaparin (Lovenox)

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Enoxaparin is a low-molecular-weight heparin that differs from unfractionated heparin by having a higher ratio of antifactor Xa to antifactor IIa.

Class Summary

Low-dose aspirin is the predominant platelet aggregation inhibitor used in children, although only limited comparative data regarding effective antiplatelet doses in pediatric populations are available.

Aspirin (Bayer Aspirin Extra Strength, Ecotrin, Aspercin)

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Aspirin is a stronger inhibitor of both prostaglandin synthesis and platelet aggregation than other salicylic acid derivatives are. The acetyl group is responsible for inactivation of cyclooxygenase via acetylation. Aspirin irreversibly inhibits platelet aggregation by inhibiting platelet cyclooxygenase. This, in turn, inhibits conversion of arachidonic acid to prostaglandin I2 (a potent vasodilator and inhibitor of platelet activation) and thromboxane A2 (a potent vasoconstrictor and platelet aggregate). Platelet-inhibition lasts for the life of the cell (approximately 10 days).

Aspirin may be used in low doses to inhibit platelet aggregation and improve complications of venous stasis and thrombosis.

Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996 Mar 1. 93(5):841-2. [QxMD MEDLINE Link]. [Full Text].
Angelini A, Calzolari V, Thiene G, et al. Morphologic spectrum of primary restrictive cardiomyopathy. Am J Cardiol. 1997 Oct 15. 80(8):1046-50. [QxMD MEDLINE Link].
Hirota Y, Shimizu G, Kita Y, et al. Spectrum of restrictive cardiomyopathy: report of the national survey in Japan. Am Heart J. 1990 Jul. 120(1):188-94. [QxMD MEDLINE Link].
Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. 1997 Jan 23. 336(4):267-76. [QxMD MEDLINE Link].
Mocumbi AO, Yacoub S, Yacoub MH. Neglected tropical cardiomyopathies: II. Endomyocardial fibrosis: myocardial disease. Heart. 2008 Mar. 94(3):384-90. [QxMD MEDLINE Link].
Webber SA, Lipshultz SE, Sleeper LA, et al. Outcomes of restrictive cardiomyopathy in childhood and the influence of phenotype: a report from the Pediatric Cardiomyopathy Registry. Circulation. 2012 Sep 4. 126(10):1237-44. [QxMD MEDLINE Link]. [Full Text].
Peddy SB, Vricella LA, Crosson JE, et al. Infantile restrictive cardiomyopathy resulting from a mutation in the cardiac troponin T gene. Pediatrics. 2006 May. 117(5):1830-3. [QxMD MEDLINE Link].
Mogensen J, Kubo T, Duque M, et al. Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations. J Clin Invest. 2003 Jan. 111(2):209-16. [QxMD MEDLINE Link].
Kaski JP, Syrris P, Burch M, et al. Idiopathic restrictive cardiomyopathy in children is caused by mutations in cardiac sarcomere protein genes. Heart. 2008 Nov. 94(11):1478-84. [QxMD MEDLINE Link].
Ware SM, Quinn ME, Ballard ET, Miller E, Uzark K, Spicer RL. Pediatric restrictive cardiomyopathy associated with a mutation in beta-myosin heavy chain. Clin Genet. 2008 Feb. 73(2):165-70. [QxMD MEDLINE Link].
Goldfarb LG, Park KY, Cervenakova L, et al. Missense mutations in desmin associated with familial cardiac and skeletal myopathy. Nat Genet. 1998 Aug. 19(4):402-3. [QxMD MEDLINE Link].
Pruszczyk P, Kostera-Pruszczyk A, Shatunov A, et al. Restrictive cardiomyopathy with atrioventricular conduction block resulting from a desmin mutation. Int J Cardiol. 2007 Apr 25. 117(2):244-53. [QxMD MEDLINE Link].
Dalakas MC, Park KY, Semino-Mora C, et al. Desmin myopathy, a skeletal myopathy with cardiomyopathy caused by mutations in the desmin gene. N Engl J Med. 2000 Mar 16. 342(11):770-80. [QxMD MEDLINE Link].
Walsh MA, Grenier MA, Jefferies JL, Towbin JA, Lorts A, Czosek RJ. Conduction abnormalities in pediatric patients with restrictive cardiomyopathy. Circ Heart Fail. 2012 Mar 1. 5(2):267-73. [QxMD MEDLINE Link].
Brodehl A, Ferrier RA, Hamilton SJ, et al, for the FORGE Canada Consortium. Mutations in FLNC are associated with familial restrictive cardiomyopathy. Hum Mutat. 2016 Mar. 37(3):269-79. [QxMD MEDLINE Link].
Wu W, Lu CX, Wang YN, et al. Novel phenotype-genotype correlations of restrictive cardiomyopathy with myosin-binding protein C (MYBPC3) gene mutations tested by next-generation sequencing. J Am Heart Assoc. 2015 Jul 10. 4(7):[QxMD MEDLINE Link].
Sanna T, Dello Russo A, Toniolo D, et al. Cardiac features of Emery-Dreifuss muscular dystrophy caused by lamin A/C gene mutations. Eur Heart J. 2003 Dec. 24(24):2227-36. [QxMD MEDLINE Link].
Imamura T, Nakazato M, Date Y, et al. Cardiac amyloidosis associated with a novel transthyretin aspartic acid-18 glutamic acid de novo mutation. Circ J. 2003 Nov. 67(11):965-8. [QxMD MEDLINE Link].
Facher JJ, Regier EJ, Jacobs GH, et al. Cardiomyopathy in Coffin-Lowry syndrome. Am J Med Genet A. 2004 Jul 15. 128(2):176-8. [QxMD MEDLINE Link].
Denfield SW, Rosenthal G, Gajarski RJ, et al. Restrictive cardiomyopathies in childhood. Etiologies and natural history. Tex Heart Inst J. 1997. 24(1):38-44. [QxMD MEDLINE Link].
Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med. 2003 Apr 24. 348(17):1647-55. [QxMD MEDLINE Link].
Russo LM, Webber SA. Idiopathic restrictive cardiomyopathy in children. Heart. 2005 Sep. 91(9):1199-202. [QxMD MEDLINE Link]. [Full Text].
Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. 2003 Apr 24. 348(17):1639-46. [QxMD MEDLINE Link].
Malcic I, Jelusic M, Kniewald H, Barisic N, Jelasic D, Bozikov J. Epidemiology of cardiomyopathies in children and adolescents: a retrospective study over the last 10 years. Cardiol Young. 2002 May. 12(3):253-9. [QxMD MEDLINE Link].
Bukhman G, Ziegler J, Parry E. Endomyocardial fibrosis: still a mystery after 60 years. PLoS Negl Trop Dis. 2008. 2(2):e97. [QxMD MEDLINE Link].
Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH. A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med. 2008 Jul 3. 359(1):43-9. [QxMD MEDLINE Link].
Cetta F, O'Leary PW, Seward JB, Driscoll DJ. Idiopathic restrictive cardiomyopathy in childhood: diagnostic features and clinical course. Mayo Clin Proc. 1995 Jul. 70(7):634-40. [QxMD MEDLINE Link].
Lewis AB. Clinical profile and outcome of restrictive cardiomyopathy in children. Am Heart J. 1992 Jun. 123(6):1589-93. [QxMD MEDLINE Link].
Arbustini E, Pasotti M, Pilotto A, Pellegrini C, Grasso M, Previtali S. Desmin accumulation restrictive cardiomyopathy and atrioventricular block associated with desmin gene defects. Eur J Heart Fail. 2006 Aug. 8(5):477-83. [QxMD MEDLINE Link].
Vaitkus PT, Kussmaul WG. Constrictive pericarditis versus restrictive cardiomyopathy: a reappraisal and update of diagnostic criteria. Am Heart J. 1991 Nov. 122(5):1431-41. [QxMD MEDLINE Link].
Ryan TD, Madueme PC, Jefferies JL, et al. Utility of echocardiography in the assessment of left ventricular diastolic function and restrictive physiology in children and young adults with restrictive cardiomyopathy: A comparative echocardiography-catheterization study. Pediatr Cardiol. 2017 Feb. 38(2):381-9. [QxMD MEDLINE Link]. [Full Text].
Choi EY, Ha JW, Kim JM, et al. Incremental value of combining systolic mitral annular velocity and time difference between mitral inflow and diastolic mitral annular velocity to early diastolic annular velocity for differentiating constrictive pericarditis from restrictive cardiomyopathy. J Am Soc Echocardiogr. 2007 Jun. 20(6):738-43. [QxMD MEDLINE Link].
Friedberg MK, Silverman NH. The systolic to diastolic duration ratio in children with heart failure secondary to restrictive cardiomyopathy. J Am Soc Echocardiogr. 2006 Nov. 19(11):1326-31. [QxMD MEDLINE Link].
Hatle LK, Appleton CP, Popp RL. Differentiation of constrictive pericarditis and restrictive cardiomyopathy by Doppler echocardiography. Circulation. 1989 Feb. 79(2):357-70. [QxMD MEDLINE Link].
Rivenes SM, Kearney DL, Smith EO, Towbin JA, Denfield SW. Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy. Circulation. 2000 Aug 22. 102(8):876-82. [QxMD MEDLINE Link]. [Full Text].
Talreja DR, Nishimura RA, Oh JK, Holmes DR. Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory. J Am Coll Cardiol. 2008 Jan 22. 51(3):315-9. [QxMD MEDLINE Link].
Yoshizato T, Edwards WD, Alboliras ET, et al. Safety and utility of endomyocardial biopsy in infants, children and adolescents: a review of 66 procedures in 53 patients. J Am Coll Cardiol. 1990 Feb. 15(2):436-42. [QxMD MEDLINE Link].
[Guideline] Cooper LT, Baughman KL, Feldman AM, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Circulation. 2007 Nov 6. 116(19):2216-33. [QxMD MEDLINE Link].
Murtuza B, Fenton M, Burch M, et al. Pediatric heart transplantation for congenital and restrictive cardiomyopathy. Ann Thorac Surg. 2013 May. 95(5):1675-84. [QxMD MEDLINE Link].
Maron BJ, Ackerman MJ, Nishimura RA, Pyeritz RE, Towbin JA, Udelson JE. Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol. 2005 Apr 19. 45(8):1340-5. [QxMD MEDLINE Link].
Weller RJ, Weintraub R, Addonizio LJ, et al. Outcome of idiopathic restrictive cardiomyopathy in children. Am J Cardiol. 2002 Sep 1. 90(5):501-6. [QxMD MEDLINE Link].
Bengur AR, Beekman RH, Rocchini AP. Acute hemodynamic effects of captopril in children with a congestive or restrictive cardiomyopathy. Circulation. 1991 Feb. 83(2):523-7. [QxMD MEDLINE Link].
Horibata Y, Murakami T, Niwa K. Effect of the oral vasopressin receptor antagonist tolvaptan on congestive cardiac failure in a child with restrictive cardiomyopathy. Cardiol Young. 2014 Feb. 24(1):155-7. [QxMD MEDLINE Link].
Zhang L, Nan C, Chen Y, et al. Calcium desensitizer catechin reverses diastolic dysfunction in mice with restrictive cardiomyopathy. Arch Biochem Biophys. 2015 May 1. 573:69-76. [QxMD MEDLINE Link].
Shaddy RE. Pulmonary hypertension in pediatric heart transplantation. Prog Pediatr Cardiol. 2000 Jun 1. 11(2):131-6. [QxMD MEDLINE Link].
Towbin JA. Cardiomyopathy and heart transplantation in children. Curr Opin Cardiol. 2002 May. 17(3):274-9. [QxMD MEDLINE Link].
Anderson HN, Cetta F, Driscoll DJ, Olson TM, Ackerman MJ, Johnson JN. Idiopathic restrictive cardiomyopathy in children and young adults. Am J Cardiol. 2018 May 15. 121(10):1266-70. [QxMD MEDLINE Link].
Fenton MJ, Chubb H, McMahon AM, et al. Heart and heart-lung transplantation for idiopathic restrictive cardiomyopathy in children. Heart. 2006 Jan. 92(1):85-9. [QxMD MEDLINE Link].
Kimberling MT, Balzer DT, Hirsch R, Mendeloff E, Huddleston CB, Canter CE. Cardiac transplantation for pediatric restrictive cardiomyopathy: presentation, evaluation, and short-term outcome. J Heart Lung Transplant. 2002 Apr. 21(4):455-9. [QxMD MEDLINE Link].
Bograd AJ, Mital S, Schwarzenberger JC, et al. Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006. Am J Transplant. 2008 Jan. 8(1):201-7. [QxMD MEDLINE Link]. [Full Text].
Al-Khaldi A, Reitz BA, Zhu H, Rosenthal D. Heterotopic heart transplant combined with postoperative Sildenafil use for the treatment of restrictive cardiomyopathy. Ann Thorac Surg. 2006 Apr. 81(4):1505-7. [QxMD MEDLINE Link].
Rao PS. Static balloon dilatation of the atrial septum. Am Heart J. 1993 Jun. 125(6):1824-7. [QxMD MEDLINE Link].
Haas NA, Laser KT, Bach S, Kantzis M, Happel CM, Fischer M. Decompressive atrioseptostomy (DAS) for the treatment of severe pulmonary hypertension secondary to restrictive cardiomyopathy. Int J Cardiol. 2016 Jan 15. 203:845-7. [QxMD MEDLINE Link].
Topilsky Y, Pereira NL, Shah DK, et al. Left ventricular assist device therapy in patients with restrictive and hypertrophic cardiomyopathy. Circ Heart Fail. 2011 May. 4(3):266-75. [QxMD MEDLINE Link]. [Full Text].
Tunuguntla H, Denfield SW, McKenzie ED, Adachi I. Mitral valve replacement for inflow obstruction of left ventricular assist device in a child with restrictive cardiomyopathy. J Thorac Cardiovasc Surg. 2016 Jan. 151(1):e11-3. [QxMD MEDLINE Link]. [Full Text].
Jaquiss RD. Ventricular assistant in restrictive cardiomyopathy: Making the right connection. J Thorac Cardiovasc Surg. 2016 Jan. 151(1):e15-6. [QxMD MEDLINE Link]. [Full Text].
Wittekind SG, Ryan TD, Gao Z, et al. Contemporary outcomes of pediatric restrictive cardiomyopathy: A single-center experience. Pediatr Cardiol. 2019 Apr. 40(4):694-4. [QxMD MEDLINE Link].

Media Gallery

Echocardiographic 4-chamber view of a child with restrictive cardiomyopathy demonstrating characteristic marked enlargement of right atrium (RA) and left atrium (LA), which are larger than left ventricle (LV).

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Sections Pediatric Restrictive Cardiomyopathy
Overview
Presentation
DDx
Workup
Treatment
Medication
References

Pediatric Restrictive Cardiomyopathy Medication

Medication Summary

Diuretic agents